Journal of Postgraduate Medicine
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Year : 1984  |  Volume : 30  |  Issue : 4  |  Page : 250-2  

Melanotic neuroectodermal tumour of the maxilla (a case report).

MV Kirtane, SB Ogale, SN Merchant, SY Sane 

Correspondence Address:
M V Kirtane

How to cite this article:
Kirtane M V, Ogale S B, Merchant S N, Sane S Y. Melanotic neuroectodermal tumour of the maxilla (a case report). J Postgrad Med 1984;30:250-2

How to cite this URL:
Kirtane M V, Ogale S B, Merchant S N, Sane S Y. Melanotic neuroectodermal tumour of the maxilla (a case report). J Postgrad Med [serial online] 1984 [cited 2022 Nov 30 ];30:250-2
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Melanotic neuroectodermal tumours are rare, peculiar to the infants and are benign with little tendency to recur after total excision or effective curettage. An unusually large and aggressive tumour of this type occurring in a 5 month old male infant is presented.


A five month old male Hindu infant was first seen by us on 6th February, 1984 with a history of a rapidly growing swelling arising from the anterior part of the upper alveolus. The tumour had been noticed when the child was 3 months old; it had been excised when it was about one cm in diameter but had recurred soon after and had rapidly progressed since then.

On examination, there was a dark brown mass, 5 cm in diameter, arising from the midline of the anterior maxilla. It was firm, friable and bled on touch. The upper lip was elevated. stretched and obscured by the tumour. The child had severe anaemia (Hb: 5.9 gm%) with resultant congestive cardiac failure. A plain radiograph showed the involved bone to be radiolucent with displacement of tooth germs. A wedge biopsy revealed a pigmented neuroectodermal tumour of the alveolus. While the anaemia was being corrected with repeated transfusions of packed cells and haematinics, the tumour grew at an alarming rate, nearly doubling its size over a span of 3 weeks [Fig. 1]. It interfered with the child's nursing and feeds had to be given through a Ryle's tube. It also compromised the airway necessitating a tracheostomy.

The tumour was excised under general anaesthesia on 6th March 1984. A sublabial incision was made on the right side, extended across the midline and the mucosa elevated. The tumour which was found to be arising from the premaxilla was freed from all sides and excised along with a small part of the premaxilla. Four hundred nil of blood was lost during surgery. The post-operative period was uneventful except for a brief period of oliguria on the third day which responded to diuretics and intravenous fluids. The child was weaned off the tracheostomy and decannulated on the twentieth post-operative day. The child has been followed up regularly and the appearance five months post-operative is shown in [Fig. 1].

Histopathological examination [Fig. 2]. revealed the typical features of this tumour-a stroma of vascularised connective tissue and tumour cells arranged in a pseudrglandular pattern. Two types of cells are seen-a large cell type with abundant pale cytoplasm containing melanin granules and pale nuclei, and a small cell type with scanty cytoplasm and dark nuclei resembling mature lymphocytes. The cells do not show plemorphism or mitotic figures.


A melanotic neuroectodermal tumour of the jaw is a rare entity, only 75 cases having been recorded in the literature till 1974.[1] It is primarily a lesion of the infant with an onset from a few weeks to 6 months after birth though a couple of cases have been recorded in adults.[3],[4] It is peculiar to the jaws, arising most often from the maxilla but has been described in the mediastinum, anterior fontanelle, shoulder, epididymis, cerebellum and the scapula. The child is generally healthy except for the tumour mass.

Controversy surrounds the pathogenesis of the tumour. The plethora of names given to it reflects the confusion, frustration and sometimes, acrimony attendant to its genesis[1]-neuroectodermal tumour of infancy, melanotic ameloblastoma, melanotic progonoma, congenital melanocarcinoma, retinal anlage tumour, pigmented epulis, pigmented teratoma etc. There are three main theories of origin: (a) that it is a congenital melanocarcinoma; (b) that it is odontogenic in origin; and (c) that it is derived from the neural crest (neuroectodermal in origin). Of these, the weight of evidence favours the neuroectodermal theory. There is abundant evidence that neural crest cells can differentiate into various cell types including melanoblasts. Also, electron microscopy has shown that the cells of this tumour appear to be of neural crest origin and their ultrastructure resembles that of melanocytes.[5] Further, it has also been shown in some cases to elaborate vanilmandelic acid (VMA)-a property shared by other neuroectodermal tumours.[2]

The lesion has generally been considered to be benign with little tendency to recur after total excision or curettage. However, Kilmer[3] has shown that these tumours have a definite potential for assuming a locally malignant character ant he recommends a radical approach with wide excision of the tissues around the tumour. Such a radical excision would be desirable and possible only if the tumour is small and easily accessible. But when the tumour is as advanced as in the case presented here it would be more rational to excise the main bulk of the tumour to restore the facial appearance and curette out the remaining tumour. If we had decided to perform a radical excision in our case, it would have involved removal of not only the premaxilla but also the hard palate and the maxilla on the left side leaving the child grossly deformed.


We are grateful to the Dean, Seth G.S. Medical College and K.F.M. Hospital for permission to use the hospital records.


1Batsakis, J. G.: Other neuroectodermal tumours and related lesions of the head and neck. Chapter
217, in, "Tumours of the Head and Neck: Clinical and Pathological Considerations," The William and Wilkins Company, Baltimore, 1974, pp. 253-254.
3Borello, E. D. and Gorlin. R. J.: Melanotic neuroectedermal tumour of infancy-a neoplasm of neural crest origin-report of a case associated with high excretion of vanilmandelic acid. Cancer, 19: 196-206, 1966.
4Kilmer, J. B.: The pigmented ameloblastomas or "retinal anlage" tumours-Report of two cases. Laryngoscope, 73: 1303-1308, 1963.
5Lurie, H. I.: Congenital melanocarcinoma, melanotic adamantinoma, retinal anlage tumour, progonoma and pigmented epulis of infancy. Cancer, 14: 1090-1108, 1961.
6Misugi, K., Okajima, H., Newton, W. A., Kmetz, D. R. and deLorimier, A. A.: Mediastinal origin of a melanotic progonoma or retinal anlage tumour: ultrastructural evidence for neural crest origin. Cancer, 18: 477-484, 1965.

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